Investigation of Thiol/Disulfide Homeostasis and Ischemia-Modified Albumin Levels in Children with Wilson Disease.

BackgroundThe aim of the present study was to assess thiol/disulfide homeostasis (TDH) parameters and ischemia-modified albumin (IMA) levels in children with Wilson Disease (WD) and to compare them to healthy controls. Methods: Based on the inclusion and exclusion criteria, fifteen children with WD and twenty-nine healthy children were enrolled, and serum thiol/disulfide and IMA levels were compared between groups. Results: The mean values of native and total thiols were significantly lower in the WD group than in the control group. The mean value of disulfide was significantly higher in the WD group than in the control group. The mean percentages of disulfide/total thiol and native thiol/total thiol were higher in the WD group than in the control group. The IMA value was also higher in the WD group than in the control group. Conclusion: The present study demonstrating altered thiol/disulfide parameters indicates increased oxidative stress in children with WD.

Salivary biomarkers in the context of gingival inflammation in children with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a life-threatening chronic inflammatory disease in children due to respiratory complications. Saliva could serve as a reservoir of bacterial colonization and potentially reflect systemic inflammation. This study investigated whether salivary triggering receptor expressed on myeloid cells 1 (TREM-1), peptidoglycan recognition protein 1 (PGLYRP1), interleukin (IL)-1ß, and calprotectin are associated with CF or reflect concomitant gingival inflammation. METHODS: Ten CF (aged 3 to 12 years) and 10 systemically healthy (SH) age- and sex-matched children (C) were enrolled in the study. Individuals with CF underwent routine laboratory determinations. Probing depth, gingival index (GI), plaque index (PI), and bleeding on probing (BOP) were recorded on fully erupted teeth and saliva samples collected. Salivary TREM-1, PGLYRP1, IL-1ß, and calprotectin were analyzed by enzyme-linked immunosorbent assay. RESULTS: Children with CF had significantly higher BOP scores (P = 0.001) and calprotectin levels (P = 0.017) compared with the C group. TREM-1, PGLYRP1, and IL-1ß could not distinguish between CF and SH but showed positive correlation with GI, PI, and BOP in both groups. Calprotectin levels positively correlated with procalcitonin (P = 0.014), thrombocyte counts (P = 0.001), mean platelet volume (P = 0.030), and with PGLYRP1 (P = 0.019) and IL-1ß (P = 0.013) in CF children. Receiver operating characteristic curve analysis for calprotectin (CFvsC) showed an area under the curve of 0.79 (95% CI 0.58 to 0.99, P = 0.034). CONCLUSIONS: CF children presented with higher gingival inflammation scores and salivary calprotectin levels, that correlated with systemic inflammatory markers. Salivary calprotectin levels were not associated with periodontal parameters. Hence, preliminary data demonstrate that salivary calprotectin might have a chairside diagnostic potential for CF in children.

A rare reason in a child with feeding intolerance: Intravaginal struvite stone.

Tokgöz Y, Erdem AO, Özbey BC, Terlemez S. A rare reason in a child with feeding intolerance: Intravaginal struvite stone. Turk J Pediatr 2018; 60: 86-88. Vaginal stones are rarely seen in childhood; they are categorized as primary and secondary whether they are a foreign object focus (nidus) or not. Urethrovaginal fistula is the most common etiological cause; other etiologies are considerably rarely reported. Primary vaginal stones are formed as a result of urinary salt accumulate. A 14-year-old girl, suffering from an unidentified neurodegenerative disease, was admitted with complaints of cough, poor feeding and vomiting. Abdominal X-ray showed a large calcific mass; further evaluation revealed a vaginal struvite stone, and it was removed surgically. No anatomical reason was determined for the formation of stone and it was accepted as primary vaginal stone.

Fat soluble vitamin levels in children with newly diagnosed celiac disease, a case control study.

BACKGROUND: In children diagnosed with celiac disease, fat soluble vitamin levels were aimed to be evaluated and it was intended to determine whether fat soluble vitamin levels were needed to be assessed routinely in these patients during diagnosis. METHODS: Between May 2015-May 2016, diagnosis symptoms of celiac patients (CD) in newly diagnosed pediatric group were questioned, fat soluble vitamin levels simultaneous with intestinal biopsies were evaluated. Vitamin levels were compared with those of healthy control group. RESULTS: A total of 52 patients involving 27 female (51.9%), 25 male (48.1%); and a total of 50 healthy control group including 25 female (50%), 25 male (50%) were evaluated. The average age of patients was 9 ± 4.3 years, and their average weight was determined as 16.2 ± 6.3 kg. Growth retardation was the most frequent symptom in our patients (61.5%). Abdominal pain (51.9%) and diarrhea (11.5%) are among the other most commonly seen symptoms. In the histological examination of patients, Marsh 3B n = 23 (45.1%) was mostly established. Vitamin A and vitamin D levels of patients were determined significantly lower compared to those of control group. Vitamin A and vitamin D deficiencies were identified significantly higher compared to those of healthy control group. Vitamin D insufficiency was observed in 48 patients (92.3%) and vitamin D deficiency was determined in 32 (61.5%) out of 48. Vitamin A deficiency was established in 17 (32.7%) patients. Vitamin E and vitamin K1 deficiency were determined in no patients. In the healthy control group, vitamin D deficiency was seen in 2 (4%) patients, vitamin D insufficiency was determined in 9 (18%) patients. Other vitamin levels were identified at normal levels in the healthy group. CONCLUSIONS: In newly diagnosed children with CD, a significant lowness was established in vitamin D and A. The evaluation of vitamin A and D levels will be helpful in the course of diagnosis in these patients.

Investigation of antiviral resistance and escape mutations in children with naive chronic hepatitis B patients and their parents.

Tokgöz Y, Terlemez S, Sayan M, Kirdar S. Investigation of antiviral resistance and escape mutations in children with naive chronic hepatitis B patients and their parents. Turk J Pediatr 2018; 60: 514-519. In this study, it was aimed to scan the resistance to nucleoside analogs in naive pediatric patients with chronic hepatitis B treatment and their parents and the rate of accompanying possible escape mutations. A total of 34 children who did not receive any treatment regarding chronic hepatitis B and 19 parents who caused vertical transmission or acquired transmission from father were involved in the study. Serological tests concerning hepatitis B virus and transaminases in conjunction with viral load were studied. HBV genotypes, subgenotypes were determined by surface gene sequencings. The gene mutations coding polymerase (pol) for resistance against nucleoside analogs and escape mutations in the genes coding surface (S) proteins were analyzed with PCR method. All cases were genotype D. Only one pediatric patient was D2; the rest of all pediatric patients and their parents were genotype D1. Resistance was not identified against nucleoside analogs in any children or their parents. HBsAg escape mutations determined in the chronic hepatitis B patients were 18.8% (10 case). It can be speculated with this results that the resistance may not be considered as a problem in the preference of nucleoside analogs in treatment of naive children. Nevertheless, escape mutations were seen as high in both children and parents as well. Since it interests public health on a large scale, advanced studies and evaluation of vaccination escape mutations` rate in broad case series and their follow up are of great importance in the determination of health policies with regard to hepatitis B infection control.

The evaluation of arterial stiffness of essential hypertension and white coat hypertension in children: a case-control study.

BACKGROUND: The aim of this study was to determine and compare cardiovascular risks by assessing arterial stiffness in children with essential hypertension and white coat hypertension. METHODS: Paediatric patients followed up with essential hypertension and white coat hypertension diagnoses and with no established end organ damage were involved in the study. Arterial stiffness in children included in the study was evaluated and compared by using the oscillometric device (Mobil-O-Graph) method. RESULTS: A total of 62 essential hypertension (34 male, 28 female), 38 white coat hypertension (21 male, 17 female), and 60 healthy controls (33 male, 27 female) were assessed in the present study. Pulse wave velocity of the essential hypertension, white coat hypertension, and control group was, respectively, as follows: 5.3±0.6 (m/s), 5.1±0.4 (m/s), 4.3±0.4 (m/s) (p<0.001); augmentation index outcomes were, respectively, determined as follows: 21.3±6.5, 19.3±6.4, 16.0±0.3 (p<0.001). Pulse wave velocity and augmentation index values of children with essential hypertension and white coat hypertension were found to be higher compared with the control group. This level was identified as correlated with the duration of hypertension in both patient groups (p<0.01). CONCLUSION: Arterial stiffness in children with essential hypertension and white coat hypertension was impaired compared with healthy children. This finding has made us think that white coat hypertension is not an innocent clinical situation. This information should be taken into consideration in the follow-up and treatment approaches of the patients.

Perilipin polymorphisms are risk factors for the development of obesity in adolescents? A case-control study.

BACKGROUND: The variations in perilipin gene (PLIN) were previously associated with obesity. We examined the association of polymorphisms at the PLIN locus in adolescents with obesity and their connection with serum adipokines. METHODS: A total of 308 children (206 obese, 66.8% and 102 healthy control, 33.2%) between the ages of 10-18 years were included into the study. PLIN gene analysis [PLIN 1, PLIN 4, PLIN 6, PLIN 5'UTR-1234 C > G and PLIN 10171 A/T] were studied by Real Time-PCR. Serum leptin, adiponectin, resistin and ghrelin levels were studied by ELISA method in both groups and their link with perilipin polymorphisms were analyzed. RESULTS: Serum leptin level was found significantly high in obese adolescents. Other adipokine levels were similar in both groups. The incidence of PLIN 1, PLIN 4, PLIN 5'UTR-1234 C > G and PLIN 10171 A/T minor and major alleles was similar in both groups. PLIN 6 T/T allele was determined significantly high in obese adolescents compared to that of control group. No correlation was detected between perilipin polymorphism and serum levels of adipokines. CONCLUSION: The PLIN 6 polymorphism of the perilipin gene may influence the risk of the obesity during adolescence. TRIAL REGISTRATION: Retrospectively registered.

Aminotransferase elevations in rotavirus positive and negative acute gastroenteritis and its relation with disease severity.

BACKGROUND: The aim of this study was to investigate the frequency of elevated alanine (ALT) and aspartate aminotransferase (AST) levels in children with rotavirus positive and negative gastroenteritis as well as the average time to normalization of liver enzymes. METHODS: Into the study 298 patients with rotavirus positive and 321 patients with rotavirus negative gastroenteritis were enrolled. RESULTS: Mean AST (56.9±2.1 and 40.2±0.9 U/L, respectively, P=0.000) and ALT (33.1±1.7 and 22.4±0.8 U/L, respectively, P=0.000) levels were significantly higher in the rotavirus positive than rotavirus negative patients. Logistic regression analysis showed that rotavirus positivity was significant independent factor for both AST and ALT elevation. Severity of gastroenteritis was another significant independent factor for ALT elevation. The average transaminase normalization time for AST and ALT levels were similar both rotavirus positive and negative groups. CONCLUSIONS: Rotavirus positivity and severity of gastroenteritis were independent risk factors for elevated ALT levels in children with gastroenteritis.

Evaluation of arterial stiffness in children with type 1 diabetes using the oscillometric method.

AIMS: Pulse wave velocity (PWV) is an accepted evaluation method to assess vascular changes and determine cardiovascular disease risk in type 1 diabetes (T1D) patients. The aim of this study was to identify atherosclerosis risk by using oscillometric device in pediatric patients who had T1D but no end organ impairment and no cardiovascular disease findings. MATERIALS AND METHODS: Pediatric patients with T1D and no determined end organ impairment and cardiovascular disease were involved in the study. RESULTS: A total of 72 patients with T1D containing 32 males and 40 females were included in the study. A total of 77 patients including 39 males and 38 females were evaluated as healthy control group. The average age of patients with T1D was 12.8±3.7years, their average weight was established as 43.8±16.7kg. The average age of control group was 12.3±1.6years and average weight was determined as 46.8±12.8kg. When the results obtained by pulse wave method were compared; PWV and Alx_75 values in T1D patients (respectively, 4.63±0.40 and 22.9±6.7) were determined significantly higher than those of control group (respectively, 4.42±0.34 and 16.6±6.6). A positive correlation was identified between diabetes duration and HbA1c (instant and mean) levels in patients with T1D with respect to PWV and Alx_75 values. CONCLUSIONS: Arterial stiffness was impaired in children with T1D with no end organ impairment using oscillometric method. This impairment was related to high HbA1c levels and diabetes duration.

Investigation of adropin and leptin levels in pediatric obesity-related nonalcoholic fatty liver disease.

AIM: Nonalcoholic fatty liver disease (NAFLD) is the accumulation of excess fat in the liver in the absence of alcohol consumption, which is commonly associated with obesity and increased risk of atherosclerosis as well as insulin resistance. Adropin is a recently identified protein encoded by the gene related with energy homeostasis, which is expressed in the liver and the brain and has a role in preventing insulin resistance and obesity. The aim of this study was to investigate the serum adropin and leptin levels in obese adolescents and compare the patients with, and without, NAFLD and with healthy controls. METHODS: Sixty-four obese adolescents (30 with NAFLD, 34 without NAFLD) and 36 healthy controls were enrolled in the study. Serum adropin and leptin levels were evaluated by sandwich enzyme-linked immunosorbent assay. RESULTS: Serum adropin levels were significantly lower in obese children than healthy controls (3.2±1.0 and 9.2±1.2 ng/mL, respectively, p=0.001). Serum leptin levels were significantly higher in patients than in controls (12.4±1.1 and 4.1±3.1 pg/mL, respectively; p=0.000). Serum adropin levels of patients with NAFLD were significantly lower than in patients without NAFLD (2.9±0.5 and 3.5±1.2 ng/mL, respectively; p=0.023) and healthy controls (p=0.000). Logistic regression analysis showed that a decrease in adropin levels was the only independent factor for fatty liver disease in obese adolescents (odds ratio: 3.07, 95% confidence interval 1.14-8.2, p=0.026). Leptin, relative weight and HOMA-IR of the patients were not independent risk factors for NAFLD. CONCLUSIONS: In this study, serum adropin levels were significantly lower in obese adolescents with fatty liver disease compared to patients without fatty liver disease and healthy controls. Lower adropin level was an independent risk factor for NAFLD in obese adolescents in logistic regression analysis. Assessment of serum adropin concentrations may provide a reliable indicator of fatty liver disease in obese adolescents.

Evaluation of serum neopterin levels and its relationship with adipokines in pediatric obesity-related nonalcoholic fatty liver disease and healthy adolescents.

AIM: Nonalcoholic fatty liver disease (NAFLD) is associated with inflammation and increased risk of atherosclerosis. Neopterin is regarded as a biochemical marker of cell-mediated immunity, which is secreted by monocytes and macrophages, mainly in response to interferon-gamma. The aim of the present study was to investigate the serum neopterin levels in obese adolescents and compare the neopterin levels in patients with and without NAFLD and also with healthy controls. The second aim of the study was to research the possible relationship between neopterin levels and adipokines (leptin, adiponectin, resistin, and ghrelin). METHODS: Ninety-three obese adolescents (39 with NAFLD, 54 without NAFLD) and 55 healthy controls were enrolled in the study. Serum levels of neopterin and adipokines were measured with high-performance liquid chromatography and sandwich enzyme-linked immunosorbent assay, respectively. RESULTS: Serum neopterin levels were significantly higher in patients with NAFLD (3.20 ± 0.09 nmol/L) than in their healthy peers (2.91 ± 0.08 nmol/L) (p=0.020). Neopterin levels were positively correlated with leptin levels in obese patients (r=0.380, p<0.001) and in the group comprising all individuals (r=0.206, p<0.05). There was no correlation between neopterin concentrations and relative weight, alanin aminotransferase, adiponectin, resistin, and ghrelin levels. CONCLUSION: The serum neopterin levels were significantly higher in obese adolescents with fatty liver disease compared to controls, and this may be related to increased cell-mediated immunity in fatty liver disease.